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Myeloproliferative disorders

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https://www.readbyqxmd.com/read/29129488/dna-hypomethylating-agents-as-epigenetic-therapy-before-and-after-allogeneic-hematopoietic-stem-cell-transplantation-in-myelodysplastic-syndromes-and-juvenile-myelomonocytic-leukemia
#1
REVIEW
Christian Flotho, Sebastian Sommer, Michael Lübbert
Myelodysplastic syndrome (MDS) is a clonal bone marrow disorder, typically of older adults, which is characterized by ineffective hematopoiesis, peripheral blood cytopenias and risk of progression to acute myeloid leukemia. Juvenile myelomonocytic leukemia (JMML) is an aggressive myeloproliferative neoplasm occurring in young children. The common denominator of these malignant myeloid disorders is the limited benefit of conventional chemotherapy and a particular responsiveness to epigenetic therapy with the DNA-hypomethylating agents 5-azacytidine (azacitidine) or decitabine...
November 9, 2017: Seminars in Cancer Biology
https://www.readbyqxmd.com/read/29128551/transplant-decisions-in-patients-with-myelofibrosis-should-mutations-be-the-judge
#2
REVIEW
Rachel B Salit, H Joachim Deeg
The prognosis of myeloproliferative neoplasms (MPN), including primary myelofibrosis (PMF), polycythemia vera (PV; post-PV MF) and essential thrombocythemia (ET; post-EMF) varies considerably, between these disorders as well as within each diagnosis. Molecular studies have identified "driver mutations", in JAK2, MPL1 and CALR, and additional somatic DNA mutations, including ASXL1, EZH2, IDH1/2 and SRSF2, that affect prognosis differentially. Patients with mutations in CALR (type1) have a better outlook than patients with mutations in JAK2 or MPL, while patients without any of the driver mutations (triple negative) have the shortest life expectancy...
November 8, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29095762/acquired-hemophilia-with-thrombosis-in-a-cancer-patient-an-unusual-presentation
#3
Senem Maral, Sule Mine Bakanay, Imdat Dilek
: Acquired hemophilia A (AHA) which presents with spontaneous severe intramuscular, mucosal and/or subcutaneous bleeding is a rare bleeding disorder. Even 50% of AHA patients are defined as idiopathic; 10% of cases are related with malignancy. Here, we present a case of AHA in a 43-year-old lady who was diagnosed with malignancy and venous thromboembolism on vena cava 2 years ago. To the best of our knowledge, this is the first report in literature presented with both acquired hemophilia and thrombosis associated with malignancy...
November 1, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29079125/hereditary-persistence-of-hemoglobin-f-is-protective-against-red-cell-sickling-a-case-report-and-brief-review
#4
Alexandra Sokolova, Anton Mararenko, Alexander Rozin, Alida Podrumar, Vladimir Gotlieb
Fetal hemoglobin (HbF) is a physiologic protein tetramer that is crucial for a developing fetus to survive in utero. Maternal hemoglobin has a relatively lower affinity for oxygen, and thus allows for an efficient transfer of oxygen from maternal to fetal blood. In addition to fulfilling a critical physiologic role, HbF is also known to alleviate symptoms of sickle-cell disease (SCD). The concentration of HbF depends on several factors. HbF is elevated in inherited conditions, such as hereditary persistence of HbF, hereditary spherocytosis, and thalassemia...
October 16, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/29078922/myeloproliferative-disorders
#5
REVIEW
Brian Meier, John H Burton
The emergency providers generally encounters myeloproliferative disorders (MPNs) in 1 of 2 ways: as striking laboratory abnormalities of seeming unknown consequence, or in previously diagnosed patients presenting with complications. The course of patients with MPNs is highly variable, but major complications can arise. Emergent conditions related to hyperviscosity need to be recognized early and treated aggressively. Rapid hydration, transfusion, cytoreduction, and early hematology consultation can be lifesaving...
December 2017: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/29076133/effects-of-vascular-endothelial-growth-factors-and-their-receptors-on-megakaryocytes-and-platelets-and-related-diseases
#6
REVIEW
Jin-Gang Yang, Li-Li Wang, Dong-Chu Ma
It is well known that vascular endothelial growth factors (VEGFs) and their receptors (vascular endothelial growth factor receptors, VEGFRs) are expressed in different tissues, and VEGF-VEGFR loops regulate a wide range of responses, including metabolic homeostasis, cell proliferation, migration and tubuleogenesis. As ligands, VEGFs act on three structurally related VEGFRs (VEGFR1, VEGFR2 and VEGFR3 [also termed FLT1, KDR and FLT4, respectively]) that deliver downstream signals. Haematopoietic stem cells (HSCs), megakaryocytic cell lines, cultured megakaryocytes (MKs), primary MKs and abnormal MKs express and secrete VEGFs...
October 26, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29046979/medical-management-of-pulmonary-hypertension-with-unclear-and-or-multifactorial-mechanisms-group-5-is-there-a-role-for-pulmonary-arterial-hypertension-medications
#7
REVIEW
Jason Weatherald, Laurent Savale, Marc Humbert
PURPOSE OF REVIEW: The purpose of this review was to outline the mechanisms and to review recent literature on pulmonary arterial hypertension (PAH) medications in group 5 pulmonary hypertension (PH). RECENT FINDINGS: The first steps in management are to understand the mechanisms and hemodynamic profile and to exclude chronic thromboembolic disease. Recent studies in the past 5 years have found that PAH medications may improve hemodynamics in patients with pre-capillary pulmonary hypertension due to sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, and myeloproliferative disorders with dasatinib-induced PH...
October 18, 2017: Current Hypertension Reports
https://www.readbyqxmd.com/read/29040257/current-challenges-in-the-management-of-essential-thrombocythemia
#8
Ariel Kleman, Arun K Singavi, Laura C Michaelis
Essential thrombocythemia (ET), an uncommon blood cancer, is one of the classic myeloproliferative neoplasms, a category that also includes polycythemia vera and primary myelofibrosis. All 3 diseases are clonal hematopoietic stem cell disorders. Since 2005, when scientists discovered a molecular aberration driving clonal hematopoiesis in polycythemia vera, our understanding of the genomic underpinnings of these conditions has increased rapidly. Over the last decades, primary prevention of thrombotic and hemorrhagic complications has improved the lives of patients with ET, and the ability to characterize the disease by the presence or absence of molecular mutations has lent precision to our prognostic models...
October 2017: Clinical Advances in Hematology & Oncology: H&O
https://www.readbyqxmd.com/read/29037480/linking-energy-sensing-to-suppression-of-jak-stat-signalling-a-potential-route-for-repurposing-ampk-activators
#9
REVIEW
Claire Speirs, Jamie J L Williams, Kirsten Riches, Ian P Salt, Timothy M Palmer
Exaggerated Janus kinase-signal transducer and activator of transcription (JAK-STAT) signalling is key to the pathogenesis of pro-inflammatory disorders, such as rheumatoid arthritis and cardiovascular diseases. Mutational activation of JAKs is also responsible for several haematological malignancies, including myeloproliferative neoplasms and acute lymphoblastic leukaemia. Accumulating evidence links adenosine 5'-monophosphate (AMP)-activated protein kinase (AMPK), an energy sensor and regulator of organismal and cellular metabolism, with the suppression of immune and inflammatory processes...
October 13, 2017: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/29036785/wide-transcriptome-analysis-and-cellularity-of-bone-marrow-cd34-lin-cells-of-patients-with-chronic-phase-chronic-myeloid-leukemia-at-diagnosis-vs-12-months-of-first-line-nilotinib-treatment
#10
Alessandra Trojani, Ester Pungolino, Giuseppe Rossi, Mariella D'Adda, Milena Lodola, Barbara Di Camillo, Alessandra Perego, Mauro Turrini, Ester Orlandi, Lorenza Borin, Alessandra Iurlo, Simona Malato, Francesco Spina, Maria Luisa Latargia, Francesco Lanza, Salvatore Artale, Michela Anghilieri, Maria Cristina Carraro, Gabriella De Canal, Enrica Morra, Roberto Cairoli
BACKGROUND: Chronic myeloid leukemia (CML) is a clonal myeloproliferative disorder with heterogeneous biological and clinical features. The biomolecular mechanisms of CML response to tyrosine-kinase inhibitors are not fully defined. OBJECTIVE: We undertook a gene expression profiling (GEP) study of selected bone marrow (BM) CD34+/lin- cells of chronic-phase CML patients at diagnosis and after 12 months of TKI nilotinib to investigate molecular signatures characterizing both conditions...
September 29, 2017: Cancer Biomarkers: Section A of Disease Markers
https://www.readbyqxmd.com/read/28991281/splanchnic-vein-thrombosis-as-a-first-manifestation-of-primary-myelofibrosis
#11
Gregorio Campos-Cabrera, Virginia Campos-Cabrera, Salvador Campos-Cabrera, José-Luis Campos-Villagómez, Alejandra Romero-González
Myeloproliferative neoplasms are chronic disorders of clonal hematopoietic stem cells, characterized by an overproduction of functional granulocytes, red blood cells and / or platelets, and one of the major complications is the occurrence of venous and arterial thrombotic problems caused by increased platelet aggregation and thrombin generation. In this study 11 cases of primary myelofibrosis (PM) were evaluated and 2 debuted with splanchnic venous thrombosis (SVT); so after seeing the results of this study and of world literature, it is suggested that in patients with SVT, diagnostic methods for PM like the JAK2V617F mutation should be included...
2017: Gaceta Médica de México
https://www.readbyqxmd.com/read/28990077/inhibitory-effect-of-the-anthelmintic-drug-pyrvinium-pamoate-on-t315i-bcr%C3%A2-abl%C3%A2-positive-cml-cells
#12
Jing Zhang, Yanli Jin, Jingxuan Pan
Chronic myeloid leukemia (CML) is a clonal myeloproliferative disorder characterized by a chromosome translocation that generates the BCR‑ABL oncogene, which encodes a constitutively activated tyrosine kinase. Despite progress in controlling CML at the chronic phase by first and second generations of BCR‑ABL tyrosine kinase inhibitors (TKIs), effective drugs with good safety are not available for CML patients harboring T315I BCR‑ABL and those in advanced stages of CML. Therefore, there is an urgent requirement for the development of effective therapies against T315I BCR‑ABL...
December 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28982745/nccn-guidelines-insights-myeloproliferative-neoplasms-version-2-2018
#13
Ruben A Mesa, Catriona Jamieson, Ravi Bhatia, Michael W Deininger, Christopher D Fletcher, Aaron T Gerds, Ivana Gojo, Jason Gotlib, Krishna Gundabolu, Gabriela Hobbs, Brandon McMahon, Sanjay R Mohan, Stephen Oh, Eric Padron, Nikolaos Papadantonakis, Philip Pancari, Nikolai Podoltsev, Raajit Rampal, Erik Ranheim, Vishnu Reddy, Lindsay A M Rein, Bart Scott, David S Snyder, Brady L Stein, Moshe Talpaz, Srdan Verstovsek, Martha Wadleigh, Eunice S Wang, Mary Anne Bergman, Kristina M Gregory, Hema Sundar
Myeloproliferative neoplasms (MPNs) are a group of heterogeneous disorders of the hematopoietic system that include myelofibrosis (MF), polycythemia vera (PV), and essential thrombocythemia (ET). PV and ET are characterized by significant thrombohemorrhagic complications and a high risk of transformation to MF and acute myeloid leukemia. The diagnosis and management of PV and ET has evolved since the identification of mutations implicated in their pathogenesis. These NCCN Guideline Insights discuss the recommendations outlined in the NCCN Guidelines for the risk stratification, treatment, and special considerations for the management of PV and ET...
October 2017: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/28981149/an-interesting-case-of-pyoderma-gangrenosum-with-immature-hystiocytoid-neutrophils
#14
Catherine Besner Morin, Benoit Côté, Annie Belisle
We present a unique case of a 36-year-old male who developed more than twenty Pyoderma gangrenosum ulcers demonstrating on histopathology a dense inflammatory infiltrate composed of histiocytoid mononuclear immature cells with a strong positivity for myeloperoxidase and Leder stain, suggesting a myeloid lineage in the absence of a concomitant myeloproliferative disorder. Histiocytoid Sweet syndrome is now recognized as a histological subtype of Sweet syndrome. Although Pyoderma gangrenosum and Sweet syndrome belong to the spectrum of neutrophilic diseases, to the best of our knowledge, this is the first case of a "Histiocytoid Pyoderma gangrenosum" encompassing immature granulocytes in the absence of leukemia cutis...
October 5, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28948488/prognostication-in-philadelphia-chromosome-negative-myeloproliferative-neoplasms-a-review-of-the-recent-literature
#15
REVIEW
Amy Zhou, Amber Afzal, Stephen T Oh
PURPOSE OF REVIEW: The prognosis for patients with Philadelphia chromosome (Ph)-negative myeloproliferative neoplasms (MPNs) is highly variable. All Ph-negative MPNs carry an increased risk for thrombotic complications, bleeding, and leukemic transformation. Several clinical, biological, and molecular prognostic factors have been identified in recent years, which provide important information in guiding management of patients with Ph-negative MPNs. In this review, we critically evaluate the recent published literature and discuss important new developments in clinical and molecular factors that impact survival, disease transformation, and thrombosis in patients with polycythemia vera, essential thrombocythemia, and primary myelofibrosis...
October 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/28928957/update-on-umbilical-cord-blood-transplantation
#16
REVIEW
Karen Ballen
Allogeneic hematopoietic cell transplant is a curative procedure for many patients with leukemia, lymphoma, myelodysplasia, myeloproliferative neoplasms, and genetic disorders. Umbilical cord blood transplantation is a graft source for patients who do not have a matched donor in their family or in the unrelated registry. It is particularly difficult for Black, Hispanic, and White patients of non-Western European background to find fully matched adult volunteer donors. An estimated 700,000 umbilical cord blood units have been donated for public use, and over 40,000 umbilical cord blood transplantations have been performed...
2017: F1000Research
https://www.readbyqxmd.com/read/28919910/chloroma-of-the-testis-in-a-patient-with-a-history-of-acute-myeloid-leukemia
#17
Mohammad Hossein Sanei, Matin Shariati
Chloroma, or granulocytic sarcoma, is a rare extramedullary solid hematologic cancer, found concomitant with acute myeloid leukemia. It is infrequently associated with other myeloproliferative disorders or chronic myelogenous leukemia. Chloroma of the testis after allogeneic bone marrow transplantation is particularly sparsely represented in the literature. It is suggested that an appropriate panel of marker studies be performed along with clinical correlation and circumspection to avoid misleading conclusions...
2017: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
https://www.readbyqxmd.com/read/28885361/angioimmunoblastic-t-cell-lymphoma-and-hypereosinophilic-syndrome-with-fip1l1-pdgfra-fusion-gene-effectively-treated-with-imatinib-a-case-report
#18
Masayo Yamamoto, Katsuya Ikuta, Yasumichi Toki, Mayumi Hatayama, Motohiro Shindo, Yoshihiro Torimoto, Toshikatsu Okumura
RATIONALE: Hypereosinophilic syndrome (HES) is a rare disorder characterized by hypereosinophilia and organ damage. Some cases of HES are caused by the FIP1L1/PDGFRA fusion gene and respond to imatinib. FIP1L1/PDGFRA-positive HES occasionally evolves into chronic eosinophilic leukemia or into another form of myeloproliferative neoplasm; however, the development of a malignant lymphoma is very rare. We present a rare case of angioimmunoblastic T-cell lymphoma (AITL) and HES with the FIP1L1/PDGFRA gene rearrangement...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28871936/xanthoma-like-skin-changes-in-an-elderly-woman-with-a-normal-lipid-profile
#19
Piotr Nockowski, Zdzisław Woźniak, Adam Reich, Joanna Maj
Dear Editor, An 83-year-old woman developed yellow-brownish infiltrates, nodules, and tumors mimicking xanthomas, mostly involving the periorbital and chest area within three months (Figure 1). She had no abnormalities in serum cholesterol or triglycerides levels. A detailed laboratory analysis revealed the presence of mild monoclonal gammopathy with a presence of immunoglobulin G (IgG) kappa light chains; however, according to hematologist consultation, it did not require medical intervention. Imaging assessment and ultrasound examination did not show any specific involvement of internal organs...
July 2017: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/28866671/autoimmune-myelofibrosis-clinical-features-course-and-outcome
#20
Caroline I Piatek, Maria E Vergara-Lluri, Vinod Pullarkat, Imran N Siddiqi, Casey O'Connell, Russell K Brynes, Donald I Feinstein
BACKGROUND: Autoimmune myelofibrosis (AIMF) is an underrecognized cause of nonmalignant bone marrow fibrosis which occurs in the presence or absence of a defined systemic autoimmune disease. Patients with AIMF present with cytopenias and autoantibodies, and have a distinctive nonclonal myelofibrosis on bone marrow examination. AIMF is distinguished from primary myelofibrosis by the absence of splenomegaly, eosinophilia, or basophilia, and the absence of abnormal myeloid, erythroid, or megakaryocytic morphology...
September 2, 2017: Acta Haematologica
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